Sherlock Daniel, Brown Nolan J, Chan Alvin Y, Campos Jessica K, Olaya Joffre
1Boston University, Boston, Massachusetts.
2Department of Neurological Surgery, University of California, Irvine, California; and.
J Neurosurg Case Lessons. 2024 Mar 4;7(10). doi: 10.3171/CASE23282.
Unlike syringomyelia, syringobulbia is not commonly observed in pediatric patients with Chiari malformation type I (CMI). Previous series have reported the incidence of syringobulbia as between 3% and 4% in these patients. Presentation is typically chronic, with the slow onset of neurological symptoms and cranial nerve (CN) palsies resulting from lower brainstem involvement. The authors report the first case of a pediatric patient with simultaneous CMI, syringobulbia, and unilateral CN VII palsy.
A 7-year-old male presented with right facial weakness in addition to headaches, ataxia, urinary incontinence, and falls. Magnetic resonance imaging revealed CMI with a syrinx of the cervicothoracic spine and syringobulbia. Posterior fossa decompression with duraplasty was performed without complications, and the patient was discharged home on postoperation day 5. At the 3-week follow-up, the patient's neurological deficits had largely subsided. At the 3-month follow-up, his CN VII palsy and syringobulbia had completely resolved.
Pediatric CMI patients with syringomyelia are at risk for developing syringobulbia and brainstem deficits, including unilateral facial palsy. However, craniocervical decompression can prove successful in treating such deficits.
与脊髓空洞症不同，脊髓空洞延髓症在小儿I型Chiari畸形（CMI）患者中并不常见。既往系列报道显示，这些患者中脊髓空洞延髓症的发病率在3%至4%之间。其表现通常为慢性，因下脑干受累导致神经症状和颅神经（CN）麻痹缓慢出现。作者报告了首例同时患有CMI、脊髓空洞延髓症和单侧CN VII麻痹的小儿患者。
一名7岁男性除头痛、共济失调、尿失禁和跌倒外，还出现右侧面部无力。磁共振成像显示CMI伴颈胸段脊髓空洞症和脊髓空洞延髓症。行后颅窝减压术及硬脑膜成形术，无并发症发生，患者于术后第5天出院。在3周的随访中，患者的神经功能缺损基本消退。在3个月的随访中，其CN VII麻痹和脊髓空洞延髓症已完全缓解。
患有脊髓空洞症的小儿CMI患者有发生脊髓空洞延髓症和脑干功能缺损（包括单侧面瘫）的风险。然而，颅颈减压术可成功治疗此类功能缺损。
